Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE): a 20-year single-center clinical and radiologic experience

Ramos, Guilherme Piovezani; Bartlett, David J.; Bledsoe, Adam C.; Fidler, Jeff L.; Sheedy, Shannon P.; Murray, Joseph A.

doi:10.1007/s00261-021-03005-y

Cited by 10 publications

(20 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Here, 20–40-year-old women having severe iron-deficiency anemia were more likely to exhibit steroid resistance. The non-responders usually had earlier disease onset and shorter intervals between the onset of initial manifestation and final diagnosis; however, submucosa fibrosis, which was considered as the marker of late phase ( 4 ), was more prevalent in this population. These data indicated that corticosteroid-resistant CMUSE patients had rapid disease progression and poor long-term prognoses.…”

Section: Discussionmentioning

confidence: 78%

“…Thus far, there is limited information on treatment experience for steroid- resistant/refractory CMUSE, and there is a risk for selection bias as cases with a positive outcome are more likely to be reported. Immunosuppressors were administered in several case reports of steroid-refractory CMUSE patients ( 4 , 11 , 13 ); however, the results were disappointing. In our cohort, four and three steroid-refractory patients received maintenance treatment with azathioprine and methotrexate, respectively; however, there were no clinical benefits.…”

Section: Discussionmentioning

confidence: 99%

“…Biologic therapy was attempted in steroid-refractory CMUSE patients and showed encouraging preliminary results; however, its long-term efficacy needs to be checked with further follow-up data ( 4 , 15 ). Surgery was required in 70% of patients with steroid- refractory CMUSE, despite which disease recurrence remained common in this population ( 4 , 16 ). Notably, the postoperative progression-free period was shorter in these patients than in those who achieved remission through medication only.…”

Section: Discussionmentioning

confidence: 99%

“…In a Korean CMUSE cohort ( 12 ), which included 12 patients who received corticosteroid treatment, the overall response rate was only of 25%. Besides, the experience with other immunomodulators, such as azathioprine, thalidomide, infliximab, and vedolizumab, is limited ( 4 , 11 , 13 ). Therefore, this study was conducted in a tertiary center in China to retrospectively evaluate the clinical phenotypes and prognosis of patients with CMUSE.…”

Section: Introductionmentioning

confidence: 99%

“…Since the first case (3) of CMUSE reported in 1964, approximately 100 cases have been documented. However, owing to limited large-scale research, there is no standard diagnosis for CMUSE thus far (4)(5)(6), Clinical diagnosis is based on manifestations, typical endoscopic and radiologic features, and histopathological examinations. Before final diagnosis, infectious, ischemic, or non-steroidal anti-inflammatory drug (NSAID) enteropathy, Crohn's disease, isolated vasculitis, and other autoimmune or small bowel malignancies should be excluded.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

What is the appropriate treatment strategy for cryptogenic multifocal ulcerative stenosing enteritis? A single-center experience from China

Yan

Cao

et al. 2022

Front. Med.

View full text Add to dashboard Cite

BackgroundThere are few reports on standard treatment and long-term prognosis in patients with cryptogenic multifocal ulcerative stenosing enteritis (CMUSE), particularly in patients in whom remission could not be induced by steroids. The aim of this study was to evaluate the treatment response and progression-free periods of patients with CMUSE and to identify the factors predictive of steroid resistance.MethodsThis was a retrospective cohort study that included 25 patients with clinically confirmed CMUSE between 1984 and 2021 from the enteropathy clinic of a tertiary care center. For statistical analyses, chi-square test or Fisher’s exact test were used for categorical variables. Survival curves were plotted using the Kaplan–Meier method.ResultsThe overall median progression-free period was 48 months (range, 1–108 months) after comprehensive therapy, and initial manifestation with severe bleeding rather than ileus was associated with the long-term efficacy. Patients with steroid resistance (N = 10, 55.6%) had poor prognosis, and non-responders had more favorable baseline clinical characteristics, with a higher percentage of female patients (60% vs. 12.5%), earlier disease onset (26.5 years vs. 39 years), rapid progression (42 vs. 108 months), severe anemia (80% vs. 50%), and hypoalbuminemia (50% vs. 0%), in accord with lymphangiectasia or angioectasia identified in pathology.ConclusionThere is no guaranteed treatment strategy in the maintenance of long-term clinical remission for CMUSE patients, particularly in whom with steroid resistance. Female patients with early symptoms onset, severe gastrointestinal hemorrhage and hypoalbuminemia seem to have poor long-term prognosis.

show abstract

Section: Discussionmentioning

confidence: 78%

Section: Discussionmentioning

confidence: 99%